Oral health-related quality of life in children and adolescents with osteogenesis imperfecta: cross-sectional study

Frank Rauch,Brendan Lee,Mang Shin Ma,Mohammadamin Najirad,Vernon Reid Sutton,Jean-Marc Retrouvey,Shahrokh Esfandiari

doi:10.1186/s13023-018-0935-y

Abstract

BackgroundOsteogenesis imperfecta (OI) affects dental and craniofacial development and may therefore impair Oral Health-Related Quality of Life (OHRQoL). However, little is known about OHRQoL in children and adolescents with OI. The aim of this study was to explore the influence of OI severity on oral health-related quality of life in children and adolescents.MethodsChildren and adolescents aged 8–14 years were recruited in the context of a multicenter longitudinal study (Brittle Bone Disease Consortium) that enrolls individuals with OI in 10 centers across North America. OHRQoL was assessed using the Child Perceptions Questionnaire (CPQ) versions for 8 to 10-year-olds (CPQ8–10) and for 11 to 14-year-olds (CPQ11–14).ResultsA total of 138 children and adolescents (62% girls) diagnosed with OI types I, III, IV, V and VI (n = 65, 30, 37, 4 and 2, respectively) participated in the study. CPQ8–10 scores were similar between OI types in children aged 8 to 10 years. In the 11 to 14-year-old group, CPQ11–14-scores were significantly higher (i.e. worse) for OI types III (24.7 [SD 12.5]) and IV (23.1 [SD 14.8]) than for OI type I (16.5 [SD 12.8]) (P < 0.05). The difference between OI types was due to the association between OI types and the functional limitations domain, as OI types III and IV were associated with significantly higher grade of functional limitations compared to OI type I.ConclusionThe severity of OI impacts OHRQoL in adolescents aged 11 to 14 years, but not in children age 8 to 10 years.

Highlights

Osteogenesis imperfecta (OI) affects dental and craniofacial development and may impair Oral Health-Related Quality of Life (OHRQoL)
In the group of children, there were no statistical differences in total scores of the CPQ8–10 or domain scores when different types of OI were compared (Table 2)
In this study we found that adolescents with OI type III had a more negative overall profile of OHRQoL when compared to OI type I

Summary

Introduction

Osteogenesis imperfecta (OI) affects dental and craniofacial development and may impair Oral Health-Related Quality of Life (OHRQoL). The aim of this study was to explore the influence of OI severity on oral health-related quality of life in children and adolescents. Osteogenesis Imperfecta (OI), known as “brittle bone disease,” is a rare heritable disorder (prevalence 8 per 100,000 people) that is characterized by recurrent fractures and, in severe cases, skeletal deformities [1]. Najirad et al Orphanet Journal of Rare Diseases (2018) 13:187 to health [5], and the term “oral health-related quality of life” (OHRQoL) focuses on physical, psychological, and social impacts of oral and orofacial conditions and disparities in oral health on overall health and QoL of individuals. The degree of the oral manifestations seem to be most severe in OI type III, as this type is associated with more severe craniofacial deformities and a higher prevalence of DI than milder forms of OI, such as OI type I and IV [7, 8]

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